Synonyms

Definition

Gastrinomas are functioning gastrin-producing tumors of the neuroendocrine cells that are located in the duodenum, pancreas, and/or “gastrinoma triangle” and cause clinical Zollinger–Ellison syndrome (ZES).

Annual incidence of ZES in the US is estimated at 1 in 2.5 million people. It may occur in sporadic and familial forms. Twenty to 25 % of ZES cases are associated with familial multiple endocrine neoplasia type 1 (MEN1; ZES-MEN1) (endocrine-related cancers; carcinoid tumor; hereditary cancer syndromes). Sporadic ZES can develop at any age, but the majority of cases are diagnosed between the fourth and sixth decade of life (mean age 50.5 years). ZES-MEN1 patients have multiple...